Surgical Management of Wilms Tumors with Intravenous Extension: A Multicenter Analysis of Clinical Management with Technical Insights.

BACKGROUND: About 5% of Wilms tumors present with vascular extension, which sometimes extends to the right atrium. Vascular extension does not affect the prognosis, but impacts the surgical strategy, which is complex and not fully standardized. Our goal is to identify elements of successful surgical management of Wilms tumors with vascular extensions. PATIENTS AND METHODS: A retrospective study of pediatric Wilms tumors treated at three sites (January 1999-June 2019) was conducted. The inclusion criterion was the presence of a renal vein and vena cava thrombus at diagnosis. Tumor stage, pre and postoperative treatment, preoperative imaging, operative report, pathology, operative complications, and follow-up data were reviewed. RESULTS: Of the 696 pediatric patients with Wilms tumors, 69 (9.9%) met the inclusion criterion. In total, 24 patients (37.5%) had a right atrial extension and two presented with Budd-Chiari syndrome at diagnosis. Two died at diagnosis owing to pulmonary embolism. All patients received neoadjuvant chemotherapy and thrombus regressed in 35.6% of cases. Overall, 14 patients had persistent intra-atrial thrombus extension (58%) and underwent cardiopulmonary bypass. Most thrombi (72%) were removed intact with nephrectomy. Massive intraoperative bleeding occurred during three procedures. Postoperative renal insufficiency was identified as a risk factor for patient survival (p = 0.01). With a median follow-up of 9 years (range: 0.5-20 years), overall survival was 89% and event-free survival was 78%. CONCLUSIONS: Neoadjuvant chemotherapy with proper surgical strategy resulted in a survival rate comparable to that of children with Wilms tumors without intravascular extension. Clinicians should be aware that postoperative renal insufficiency is associated with worse survival outcomes.

A Ten-year Single-center Surgical Experience With Symptomatic Complete Vascular Rings.

BACKGROUND: Limited data exist on the management of complete vascular rings (CVR) in adults. We reviewed our institution's surgical experience in the management of these patients. METHODS: Between 2010 and 2019, all adult patients that underwent a thoracotomy for a CVR repair were identified. We performed a retrospective medical record review of these patients to characterize their demographics and outcomes. RESULTS: Among the 5 patients identified (3 females, 2 males; Mean age 50 ± 9 years), anatomic variants were right arch and Kommerell diverticulum (KD) in 3 (60%) and double aortic arch in 2 (40%) patients. Indications for operation included dysphagia in 4 (80%), respiratory symptoms in 3 (60%) and aneurysmal KD in 1 (20%) patient. Two right aortic arch exclusion, 1 ligamentum arteriosum (LA) division, 1 LA division combined with a KD resection and 2 aortic reconstructions with interposition Dacron graft under partial cardiopulmonary bypass, were performed. Two carotid-subclavian artery transpositions prior to the thoracotomy were done. The postoperative length of stay was 10.0 (IQR 7.3-14.8) days. One reoperation for chylothorax and 1 for symptoms recurrence were performed for the same patient. Over a follow-up period of 1.4 (IQR 0.4-7.0) years, no mortality or major postoperative complications occurred. At their last follow-up visit, all patients reported no related remaining symptoms, except for persisting mild asthma in 1 patient. CONCLUSIONS: Open repair of CVR in adults can be performed safely with low complication rate. Symptoms improved in all patients after definitive repair.

Critical Congenital Heart Diseases in Preterm Neonates: Is Early Cardiac Surgery Quite Reasonable?

Prematurity is a recognized risk factor for morbidity and mortality following cardiac surgery. Postoperative and long-term outcomes after cardiac surgery performed in the preterm period are poorly described. The aim of this study was to analyze a population of preterm neonates operated on for critical congenital heart disease (CHD) before 37 weeks of gestational age (wGA) with special attention given to early and late mortality and morbidity. Between 2000 and 2013, 28 preterm neonates (median gestational age (GA) 34.3 weeks) underwent cardiopulmonary bypass (CPB) surgery for critical CHD before 37 wGA; records were retrospectively reviewed. All patients except three with single ventricle physiology had a single-stage anatomic repair. Overall mortality was 43 % (95 % CI 25-62). Risk factors for death were birth weight (p = 0.032) and weight at surgery (p = 0.037), independently of GA, preoperative status, CPB and aortic clamp time. Seven patients, including those with univentricular hearts, died during the postoperative period, and five in the first year after surgery. Median follow-up was 5.9 years (range 1 month-12.8 years). Kaplan-Meier survival rate was 75 % (95 % CI 59-91) at 1 month, and 57 % (95 % CI 39-75) at 1 and 5 years. Eight patients required reoperations after a delay of 2.8 ± 1.3 months; eight had bronchopulmonary dysplasia. At the end of follow-up, nine patients were asymptomatic. One-stage biventricular repair for critical CHD on preterm neonates was feasible. Mortality remained high but acceptable, mainly confined to the first postoperative year and related to small weight. Despite reoperations, long-term clinical status was good in most survivors. Further long-term prospective investigations are necessary to evaluate neurodevelopmental outcomes.

Scimitar syndrome with vein stenosis in an infant.

Scimitar syndrome is characterised by right lung hypoplasia and abnormal pulmonary venous return, known as the 'scimitar vein'. We report the case of an infant girl with scimitar syndrome who developed a severe respiratory distress mimicking asthma. Pulmonary hypertension (PH) was diagnosed, attributed to scimitar vein stenosis and a left-to-right shunt. Scimitar vein stenosis, a rare complication of scimitar syndrome, can lead to severe PH, highlighting the importance of prompt management in specialised care centres.

Aortic Valve Engineering Advancements: Precision Tuning with Laser Sintering Additive Manufacturing of TPU/TPE Submillimeter Membranes.

Synthetic biomaterials play a crucial role in developing tissue-engineered heart valves (TEHVs) due to their versatile mechanical properties. Achieving the right balance between mechanical strength and manufacturability is essential. Thermoplastic polyurethanes (TPUs) and elastomers (TPEs) garner significant attention for TEHV applications due to their notable stability, fatigue resistance, and customizable properties such as shear strength and elasticity. This study explores the additive manufacturing technique of selective laser sintering (SLS) for TPUs and TPEs to optimize process parameters to balance flexibility and strength, mimicking aortic valve tissue properties. Additionally, it aims to assess the feasibility of printing aortic valve models with submillimeter membranes. The results demonstrate that the SLS-TPU/TPE technique can produce micrometric valve structures with soft shape memory properties, resembling aortic tissue in strength, flexibility, and fineness. These models show promise for surgical training and manipulation, display intriguing echogenicity properties, and can potentially be personalized to shape biocompatible valve substitutes.

A case report of an acute coronary syndrome in a 10-year-old boy with homozygous familial hypercholesterolaemia.

BACKGROUND: Familial hypercholesterolaemia is a well-known disorder, but clinical diagnoses tend to be delayed. Acute coronary syndrome may occur in childhood. CASE SUMMARY: Our patient, a young boy with homozygous familial hypercholesterolaemia, complained of persistent chest pain at rest and suffered a non-ST-elevation myocardial infarction (NSTEMI). The diagnosis of NSTEMI was made on the basis of his clinical features, dynamic electrocardiogram changes, troponin elevation, and cardiac computed tomography findings. The patient was managed surgically by intrathoracic artery (ITA) bypass graft. During post-operative follow-up, the young patient suffered from angina pectoris from unexpected and exceptional atheroma stenosis on the ITA. DISCUSSION: Familial hypercholesterolaemia needs to be identified quickly in young patients and lipid lowering therapies should be started without delay.

Percutaneous balloon atrial septostomy on top of venoarterial extracorporeal membrane oxygenation results in safe and effective left heart decompression.

BACKGROUND: Transcatheter techniques are emerging for left atrial (LA) decompression under venoarterial extracorporeal membrane oxygenation (VA-ECMO). We aimed to assess whether balloon atrioseptostomy (BAS) is a safe and efficient strategy. METHODS: All patients who underwent percutaneous static BAS under VA-ECMO at four tertiary institutions were retrospectively reviewed. RESULTS: From 2000 to 2014, BAS was performed in 64 patients (32 adults and 32 children). Indications for ECMO support included acute myocarditis (31.3%) and non-myocarditis cardiac disease, mostly end-stage dilated cardiomyopathy (32.8%). BAS was required because of pulmonary oedema/haemorrhage and left ventricular (LV) distension. The mean balloon diameter was 21.8 ± 8.4mm. Adequate LA decompression was achieved in all patients. Mean LA pressure fell from 24.2 ± 6.9 mmHg to 7.8 ± 2.6 mmHg ( p < 0.001). The left-to-right atrial pressure gradient fell from 17.2 ± 7.1 mmHg to 0.09 ± 0.5 mmHg ( p < 0.001). Echocardiography showed an unrestrictive left-to-right atrial shunting in all patients. Improvement of day 1 chest X-ray was observed in 76.6% of patients, clinical status in 98.4% of patients and pulmonary haemorrhage in 14 out of 14 patients. Complications occurred in 9.4% of patients, representing pericardial effusion, fast atrial fibrillation, ventricular fibrillation requiring defibrillation, transient complete heart block and femoral venous dissection requiring covered stent placement. In the 37 (57.8%) patients who were successfully decannulated, the median ECMO duration was 9 (range: 4-24) days. After a median follow-up of 12.3 (range: 0.1-142) months, 35.9% patients died, 17.2% received a LV assist device as a bridge to transplantation, 31.2% were transplanted and 56.2% were home discharged and alive. CONCLUSIONS: Percutaneous BAS may be a safe and efficient strategy for discharging the LA in both adults and children supported by VA-ECMO.

[Extracorporeal life support and heart-lung transplant in children]. / Assistance circulatoire et transplantation d'organes thoraciques chez l'enfant.

Extracorporeal life support and heart and/or lung transplant are the last resort in children with end-stage cardiac and/or pulmonary failure and short-term life threaten. Currently, circulatory support is used as a bridge to recovery or as a bridge to transplant but not as a destination therapy. The Excor Berlin Heart is the long-lasting external pneumatic ventricular assist system that is currently available from infancy to adulthood. Long-term prognosis after pediatric cardiac and/or pulmonary transplant is conditioned by the occurrence of graft failure, coronary disease of the cardiac graft, viral infections and bronchiolitis obliterans of the pulmonary graft, the incidence of which increase with time. The scarcity of grafts and the risk of acute rejection due to lack of compliance with immunosuppressive treatment require the transplant specialized teams to choose the best candidates according to psychosocial and biological criteria. The next expected developments concern mainly long-term ventricular assistance with systems that allow for greater autonomy and a return to the child's home.

Standard and Strain Measurements by Echocardiography Detect Early Overloaded Right Ventricular Dysfunction: Validation against Hemodynamic and Myocyte Contractility Changes in a Large Animal Model.

BACKGROUND: Early detection of right ventricular (RV) failure is required to improve the management of patients with congenital heart diseases. The aim of this study was to validate echocardiography for the early detection of overloaded RV dysfunction, compared with hemodynamic and myocyte contractility assessment. METHODS: Using a porcine model reproducing repaired tetralogy of Fallot, RV function was evaluated over 4 months using standard echocardiography and speckle-tracking compared with hemodynamic parameters (conductance catheter). Sarcomere shortening and calcium transients were recorded in RV isolated myocytes. Contractile reserve (ΔEmax) was assessed by ß-adrenergic stimulation in vivo (dobutamine 5 µg/kg) and ex vivo (isoproterenol 100 nM). RESULTS: Six operated animals were compared with four age- and sex-matched controls. In the operated group, hemodynamic RV efficient ejection fraction was significantly decreased (29.7% [26.2%-34%] vs 42.9% [40.7%-48.6%], P < .01), and inotropic responses to dobutamine were attenuated (ΔEmax was 51% vs 193%, P < .05). Echocardiographic measurements of fraction of area change, tricuspid annular plane systolic excursion, tricuspid annular peak systolic velocity (S') and RV free wall longitudinal systolic strain and strain rate were significantly decreased. Strain rate, S', and tricuspid annular plane systolic excursion were correlated with ΔEmax (r = 0.75, r = 0.78, and r = 0.65, respectively, P < .05). These alterations were associated in RV isolated myocytes with the decrease of sarcomere shortening in response to isoproterenol and perturbations of calcium homeostasis assessed by the increase of spontaneous calcium waves. CONCLUSIONS: In this porcine model, both standard and strain echocardiographic parameters detected early impairments of RV function and cardiac reserve, which were associated with cardiomyocyte excitation-contraction coupling alterations.

Early clinical results of the telemetric adjustable pulmonary artery banding FloWatch-PAB.

BACKGROUND: Adjustment of pulmonary artery banding (PAB) may be a challenging procedure in complex congenital heart defects. Whatever the technique used, subsequent re-operations are frequently needed to control the pulmonary blood flow or pressures. OBJECTIVE: To report the efficacy of a new telemetric adjustable PAB (FloWatch-PAB) operated with the help of an external control unit that transmits to the implant energy and commands to further narrow or release the pulmonary artery using radiofrequency waves. METHODS AND RESULTS: In a multicenter, prospective, nonrandomized, single-arm clinical investigation, 13 children (median age, 4.5 months; range, 6 days to 11 years; median weight, 4.2 kg; range, 3.1 to 27 kg) underwent implantation of the FloWatch-PAB through median sternotomy in 8 and left thoracotomy in 5. The diagnosis was multiple ventricular septal (VSD) defects with complex anatomy in 3, single ventricle without pulmonary stenosis in 2, VSD with elevated pulmonary vascular resistance (PVR) in 2, atrio-ventricular canal (AVC) with elevated pulmonary vascular resistance in 1, AVC with diminutive right ventricle in 1, complex transposition of the great arteries in 3, and pulmonary atresia with complex pulmonary arteries anatomy in 1. All patients had normosystemic systolic pulmonary artery pressure. Additional procedures were performed in 7: atrial septectomy in 2, double aortic arch division in 1, patent ductus arteriosus ligation in 2, and coarctation repair in 2. There were no early or late deaths or device-related complications in a mean follow-up of 24 weeks (range, 18 to 42 weeks). A mean of 5.8 telemetric regulations per patient using the FloWatch-PAB were required to adjust the tightening of the PAB to the clinical needs (narrowing 74%, releasing 26%). At last follow-up, systolic pulmonary artery pressure was within normal range in all patients but 1. Systemic oxygen saturation demonstrated optimal regulation of the pulmonary blood flow in all according to each specific defect. Four patients were successfully corrected (VSD closure, AVSD repair, and 2 arterial switches with VSD closure). The device was easily removed and the pulmonary artery re-expanded spontaneously. CONCLUSIONS: This new device is safe and allows optimal adjustment of PAB in complex heart defects. In children requiring PAB, the use of this technology can obviate the need for early re-operations and appears to be a valuable option in the panel of surgical alternatives for selected infants.

Noncultured cell transplantation in an ovine model of right ventricular preparation.

OBJECTIVE: Beyond the first 2 months of life, pulmonary artery banding is warranted before two-stage arterial switch operation. The aim of this study was to explore whether myogenic cell transplantation could contribute to right ventricular function during pulmonary artery constriction in an ovine model. METHODS: Sixteen rams were assigned to one of the following groups: group 1, simple pulmonary artery banding (n = 5); group 2, pulmonary artery banding and cell implantation in the right ventricle (n = 7); and group 3, pulmonary artery banding and placebo injection in the right ventricle (n = 4). Hemodynamic assessment with pressure-volume loops was performed on days 0 and 60. The pulmonary artery banding and the injections were achieved through a left fourth intercostal thoracotomy. Autologous myogenic cell implantation was carried out with a noncultured cell preparation, as previously described by our group. Implanted sites were processed with monoclonal antibodies to a fast skeletal-specific isoform of myosin heavy chain (MY32). RESULTS: Skeletal myosin heavy chain expression was detected at 2 months after noncultured cell implantation in all grafted animals. Right ventricular training resulted in statistically significant increased signs of contractility in all three groups. There was no observed difference, however, between the cell therapy group and the other two groups with respect to signs of cardiac function. CONCLUSION: Successful engraftment of noncultured cells into right ventricular myocardium did not translate into a functional benefit that we could demonstrate in our ovine model. Cellular therapy thus is probably not useful to strengthen a left ventricle being retrained through pulmonary artery banding before arterial switch operation. However, cell transplantation may affect the outcome of right ventricular failure long term after atrial switch operation. Although preliminary, this investigation paves the way for further research into cellular cardiomyoplasty, right ventricular failure, and congenital heart disease.

Tissue Doppler echocardiographic and color M-mode estimation of left atrial pressure in infants.

OBJECTIVES: Using recorded flow and tissue Doppler, we evaluated the relation of peak velocity of early transmitral Doppler filling (E)/early diastolic velocity of the lateral mitral annulus (Ea) ratio and of E/flow propagation velocity (Vp) ratio to mean left atrial pressure in infants after surgery for congenital heart disease. DESIGN: Experimental design. SETTING: Pediatric intensive care unit. PATIENTS: Thirty-seven infants aged 4 (3-8) months. INTERVENTIONS: Patients underwent postoperative invasive hemodynamic monitoring with simultaneously obtained Doppler measurements. MEASUREMENTS AND MAIN RESULTS: Values are expressed as median (25th-75th percentiles). Heart rate was 145 (135-157) beats/min. Left atrial pressure was 10 (8-12) mm Hg with E/Ea 16 (12-19) and E/Vp 1.9 (1.3-2.4). E/Ea and E/Vp ratios were higher in patients with left atrial pressure >10 mm Hg (n = 18), than in patients with left atrial pressure < or =10 mm Hg (n = 19) (E/Ea, 16 [15-25] vs. 12 [9-17], p = .01; E/Vp, 2.3 [1.9-2.8] vs. 1.4 [1-1.9]. respectively, p = .001). At a cutoff point of 15, E/Ea sensitivity for left atrial pressure >10 mm Hg was 17 of 18 (94%) with specificity 13 of 18 (72%). At a cutoff point of 2, E/Vp sensitivity for left atrial pressure >10 mm Hg was 15 of 18 (83%) with specificity 16 of 18 (89%). Areas under the receiver operating characteristic curves were 0.76 (E/Ea) and 0.83 (E/Vp). CONCLUSIONS: Doppler ratios might be considered as promising noninvasive tools for left atrial pressure evaluation in infants after cardiac surgery.

Right ventricular failure secondary to chronic overload in congenital heart diseases: benefits of cell therapy using human embryonic stem cell-derived cardiac progenitors.

OBJECTIVE: Despite the increasing incidence of right ventricular (RV) failure in adult patients with congenital heart disease, current therapeutic options are still limited. By contrast to left-heart diseases, cell-based myocardial regeneration applied to the right ventricle is poorly studied, even though it may be a therapeutic solution. As human embryonic stem cell-derived cardiac progenitors seem to be good candidates owing to their proliferation capacity, our aim was to assess, in a large animal model of overloaded RV dysfunction, the feasibility and effects of such a cell therapy. METHODS: Human MesP1(+)/SSEA-1(+) cardiogenic mesodermal cells were administered using multiple intramyocardial injections 4 months after a surgical procedure mimicking the repaired tetralogy of Fallot, and their effects were observed 3 months later on hemodynamic, rhythmic, and histologic parameters. RESULTS: All pigs (sham n = 6, treated n = 6) survived without complication, and cell therapy was clinically well tolerated. Although functional, contractility, and energetics parameters evolved similarly in both groups, benefits regarding arrhythmic susceptibility were observed in the treated group, associated with a significant decrease of peri-myocyte fibrosis (5.71% ± 2.49% vs 12.12% ± 1.85%; P < .01) without interstitial fibrosis change (5.18% ± 0.81% vs 5.49% ± 1.01%). Such a decrease could be related to paracrine effects, as no human cells could be detected within the myocardium. CONCLUSIONS: Cell therapy using intramyocardial injections of human MesP1(+)/SSEA-1(+) cardiogenic mesodermal cells seems to have benefits regarding overloaded RV tissue remodeling and arrhythmic susceptibility, but this mode of administration is not sufficient to obtain a significant improvement in RV function.

Interruption of patent ductus arteriosus in children: robotically assisted versus videothoracoscopic surgery.

OBJECTIVES: If robotic surgery is to be widely used, the risks must be equivalent to those of standard techniques. This study analyzes the feasibility, safety, and efficiency of a robotically assisted technique for patent ductus arteriosus closure and compares the results with those of the videothoracoscopic technique. METHODS: During 2000, 56 children weighing 2.3 to 57 kg (mean, 12 kg) underwent surgical closure of a patent ductus arteriosus. They were distributed into 2 groups: 28 patients (group 1) underwent the videothoracoscopic technique, and 28 (group 2) underwent a robotically assisted (Zeus; Computer Motion, Inc, Goleta, Calif) approach. Operative and postoperative surgical data were studied. RESULTS: Operative time was significantly higher in the robotically assisted group. One conversion in videothoracoscopy was necessary, but no thoracotomy was required. Three persistent shunts were detected at postoperative echocardiography and were treated by means of application of a new clip with videothoracoscopy (1 in group 1 and 2 in group 2). No permanent laryngeal nerve injury and no hemorrhage were noted. The mean hospital stay was 3 days in both groups. CONCLUSIONS: Robotically assisted closure of a patent ductus arteriosus is comparable with closure by means of the videothoracoscopic technique. However, it requires a longer operative time because of the increment in complexity.

Obstructed right pulmonary venous drainage and ipsilateral lung hypoplasia.

A case of right lung hypoplasia and dyspnea, presented at our institution for cardiac evaluation, is described. Clinical evaluation and instrumental findings of normally connected but completely obstructed right pulmonary venous drainage have led us to diagnose an exceptional variant of subtotal cor triatriatum. Differential diagnosis was mainly carried out between pulmonary venous anomalies and drainage obstructions. Symptom relief was obtained with surgical excision of the atrial dividing membrane.

Paediatric video-assisted thoracoscopic clipping of patent ductus arteriosus: experience in more than 700 cases.

OBJECTIVE: To overcome drawbacks of thoracotomy and percutaneous techniques, we have performed video-assisted thoracoscopic surgery (VATS) since 1991 to treat patent ductus arteriosus (PDA). This retrospective study aims to analyse morbidity and outcome in order to add data in the evaluation of minimally invasive operations. METHODS: From September 1991 to March 2003, 703 patients underwent PDA clipping by VATS. The indications for operation were clinically significant patency or failure to close in older children. Diameter >8 mm, previous thoracotomy, calcifications, infection, or aneurysm were contraindications to VATS. In right decubitus, only two thoracostomy incisions (for 5-mm trocars) and a smaller one (for direct hook insertion) were required. In the post-operative stay two echocardiographic controls were scheduled, the first before extubation and the second before discharge. Referring cardiologists assured follow-up. RESULTS: Mean age was 3.0+/-3.8 years (5 days-33 years), mean weight 10.7+/-8.0 kg (1.2-65 kg), and 3.1% of our activity were low birth-weight infants (LBWIs) weighting < or =2.5 kg. Operative and 30-day mortality was nil. Median operative time was 20 min and median stay 2 days. Unfavourable events globally occurred in 6.8% of cases (13.6% of the LBWIs, RR 4.0, CL 95% 1.5-10.4). Recurrent laryngeal nerve injury was noted in 3% (13.6% of the LBWI, RR 5.1, CL 95% 1.6-15), but only 0.4% had long lasting dysfunction. Incidence of chylothorax was 0.6%, thoracotomy 1%, transfusion for bleeding 0.1%, pneumothorax 1.3%. LBWIs were at increased risk for the latter two events. Residual patency was detected immediately in 1.4% (all non-LBWI) and underwent additional surgery. Incidence of residual patency at follow-up was 0.6% (0% LBWI, 0.6% in 2.5-25 kg group, 5.0% in >25 kg group, P=0.001). CONCLUSIONS: This study records a long experience of PDA treatment in a wide range of body size and age. VATS clipping is safe, but LBWIs are at augmented risk of complication. It may be carried out with a high degree of efficacy in all the ductus diameters <9 mm. VATS clipping requires minimal operating time and avoids morbidity related to chest wall trauma, percutaneous vascular access, and intravascular foreign bodies.

Beating heart revascularization with minimal extracorporeal circulation in patients with a poor ejection fraction.

BACKGROUND: Coronary artery bypass grafting with cardioplegia in patients with a low ejection fraction carries a risk of myocardial ischemia. Beating heart surgery is associated with hemodynamic changes when the heart is manipulated. We assessed an alternative: minimal extracorporeal circulation for coronary artery bypass grafting on a beating heart in patients with a poor ejection fraction. METHODS: From January 2000 to January 2002, 50 patients with an ejection fraction of less than 35%, who represented 10% of all patients undergoing coronary artery procedures, underwent revascularization on a beating heart with assistance. We used a closed cardiopulmonary bypass system with a centrifugal pump without reservoir, and the surgical strategy was modified to avoid aortic cross-clamping and to decrease bypass time. RESULTS: The main preoperative characteristics were: age (mean +/- SD) of 64 +/- 11.2 years (range, 41-87 years), 35 male patients (70%), mean left ejection fraction of 24.8% +/- 11.2%, and a mean EuroSCORE of 5.8 +/- 2.7. Revascularizations of 146 distal anastomoses (2.9 +/- 0.7 grafts/patient) were completed. Twelve percent were double bypass, 86% were triple bypasses, and 2% were quadruple bypasses; the mean bypass time was 64.2 +/- 26.2 minutes. The mean graft number was 2.9, and the hospital mortality was 2%. Perioperative hematocrit levels were 30.1%, and 26% of patients received transfusions. Postoperative data showed a median extubation time of 9 hours, a median intensive care unit stay of 48 hours, and a hospital stay of 8 +/- 2 days. Postoperative complications included inotropic support (14%), cerebrovascular events (2%), reoperation for homeostasis (4%), delayed sternal closure (2%), and mediastinitis (2%). Peak troponin Ic level remained a low 2.4 +/- 1.9 g/mL. Follow-up at 6 months was complete with 1 late mortality and with a mean ejection fraction of 30.5% +/- 10.8% for the survivors. CONCLUSIONS: Coronary revascularization on a beating heart with extracorporeal assistance can be done in patients with a low ejection fraction. It avoids the myocardial injury associated with aortic cross-clamping and allows safe and complete coronary revascularization.

The modified Fontan procedure with use of extracardiac conduit in adults: analysis of 32 consecutive patients.

BACKGROUND: Uncommonly, adults with functionally univentricular hearts are becoming candidates for a Fontan procedure. The purpose of this study was to evaluate the course of patients undergoing the modified Fontan procedure with an extracardiac conduit in recent years. METHODS: Between January 2003 and December 2013, 32 adult patients (17 female and 15 male) underwent total cavopulmonary connection (TCPC) with extracardiac conduit. The median age at procedure was 24.5 years (interquartile range [IQR] 20 to 33 years). The diagnoses included double-inlet left ventricle (DILV) in 10 patients (31.2%), tricuspid atresia in 8 patients (25%), double-outlet right ventricle in 4 patients (12.5%), heterotaxia in 4 patients (12.5%), and mitral atresia in 2 patients (6.2%). Seventy-eight percent of patients had undergone at least one prior palliative procedure; the most common procedures were Blalock-Taussig shunt (16 patients), superior cavopulmonary shunt (12 patients), and pulmonary artery banding (6 patients). All patients underwent cardiac catheterization preoperatively. Aortic cross-clamping was necessary in 15 patients for intracardiac procedures. Fenestration was required in 9 patients (28%). Four concomitant intraoperative cryoablation procedures were performed. RESULTS: There was no hospital mortality. One patient (3.1%) died 6 months after undergoing TCPC. Morbidities included prolonged pleural effusion lasting more than 7 days in 20 patients (62.5%), atrial arrhythmias in 4 patients (12.5%), and permanent pacemakers in 3 patients (9.3%). The median follow-up time was 33 months (interquartile range [IQR], 10.5 to 50 months). Actuarial survival was 91.83% (95% confidence limits, 71.07 to 97.89) at 1 year and 5 years. Ninety-two percent of patients were in New York Heart Association class I or II at follow-up. The median postoperative oxygen saturation was 95% (IQR, 93% to 95.5%). Cardiac arrhythmia occurred in 4 patients. Systolic ventricular function improved during follow-up for all patients except 1 patient, who underwent cardiac transplantation 7 months after the TCPC. CONCLUSIONS: The modified Fontan procedure with use of an extracardiac conduit can be performed in adults with encouraging early and midterm results. The majority of late survivors had improved quality of life. The incidence of late death, reoperation, arrhythmias, and thromboembolic events was low during follow-up.

Reoperations for left atrioventricular valve dysfunction after repair of atrioventricular septal defect.

OBJECTIVES: Postoperative left atrioventricular valve (LAVV) dysfunction is known to be the principal risk factor influencing outcome after repair of all types of atrioventricular septal defect (AVSD). The purpose of the present study was to identify the risk factors for reoperation and to assess the outcomes after reoperation for LAVV dysfunction. METHODS: Records of 412 patients who underwent anatomical repair for different types of AVSD from January 2000 to July 2012 were reviewed. The study group (n = 60) included 13 additional patients for whom repair ± LAVV reoperation was performed in a primary institution. Outcomes, independent risk factors, reoperation and death were analysed. RESULTS: There were 7 early, (1.7%) and 1 late death. Forty-seven (11.4%) required 64 reoperations for LAVV dysfunction. The median delay for the first LAVV reoperation was 3.5 months (range: 5 days to 10.0 years). Unbalanced ventricles with small left ventricle [odds ratio (OR) = 4.06, 95% confidence interval (CI): 1.58-10.44, P = 0.004], double-orifice LAVV (OR = 5.04, 95% CI: 1.39-18.27, P = 0.014), prior palliative surgery (OR = 3.5, 95% CI: 1.14-10.8, P = 0.029) and discharge echocardiography documenting LAVV regurgitation grade >2 (OR = 21.96, 95% CI: 8.91-54.09, P < 0.001) were found to be independent risk factors for LAVV reoperation. Twelve-year survival and freedom from LAVV reoperation rates were, respectively, 96.1% (95% CI: 94.1-98.1) and 85.8% (95% CI: 81.3-90.3). Survival was significantly worse in patients who underwent LAVV reoperation (P < 0.001) and in those who underwent valve replacement vs valve repair (P = 0.020). CONCLUSION: After AVSD repair, LAVV dysfunction appears to be the principal factor that influences outcome. It can usually be managed by repair. Need for multiple reoperations is not uncommon. Long-term outcome in patients with repaired LAVV is favourable.